Radiology: Ulrike Löbel, Brigitte Bison
Surgery: Gurish Solanki
Pathology: Christian Thomas; Martin Hasselblatt
Oncology: Jenny Adamski, Iwona Filipek, Miklos Garami, Michal Zapotocky, Ofelia Cruz, Uwe Kordes
Radiotherapy: Beate Timmerman
Choroid plexus tumours (CPT) are rare brain tumors of the choroid plexus epithelium accounting for 1–4% of all pediatric brain tumors. WHO grading differentiates between benign choroid plexus papilloma (CPP, °I), intermediate atypical choroid plexus papilloma with increased mitotic activity (APP, °III) or malignant choroid plexus carcinoma (CPC, °III) with frank signs of malignancy. APP and CPC have a peak incidence in the first year of life. Methylation analysis may segregate these groups and help risk stratification, as some CPP and APP cluster with CPCs. Li-Fraumeni syndrome occurs in at least 30% of CPC and may be detected in all CPT.
The CPT-SIOP-2000 trial (NCT00500890) established an international registry for all patients with CPT as well as an interventional trial for patients requiring post-operative non-surgical treatment. The international CPT-SIOP registry (www.uke.de/cpt) is curating a database for these patients and is open for all new patients of any age with CPT.
Based on the CPT-SIOP experience our working group has established a risk-adapted treatment guidance and offers reference review for radiology and pathology, as well as clinical advice. We have an ongoing imaging and biology project and are evaluating novel therapeutic approaches.
Safe staged maximum resection is important for all CPTs. Treatment results with Carboplatin/ Etoposide/Vincristine based chemotherapy with focal radiotherapy in CPC patients older than three years are superior to historical controls. A subset of young CPC is cured by surgery and conventional chemotherapy, without irradiation or high-dose chemotherapy.